Chest
Clinical InvestigationsMiscellaneousPulmonary Arteriovenous Malformations: Screening Procedures and Pulmonary Angiography in Patients With Hereditary Hemorrhagic Telangiectasia
Section snippets
Materials and Methods
All HHT patients identified during a comprehensive epidemiologic survey in the county of Fyn, Denmark (population approximately 500,000)7 and their first-degree relatives (age > 18 years) were offered screening for PAVM with contrast echocardiography (CE). The HHT diagnosis was based on the following: (1) presence of multiple (> 15) telangiectatic lesions; and (2) either a family history of bleeding or recurrent episodes of bleeding. The criterion of heredity was considered to be met if the
Study Population
Twenty-five patients (14 men and 11 women) were included in the study and had PA performed (mean age, 44 years; range, 25 to 75 years). All fulfilled the diagnostic criteria for HHT and had positive findings on CE.
In 15 patients (8 men and 7 women), at least one PAVM was demonstrated at PA. Twelve of these patients had PAVMs with feeding vessels > 3 mm and were referred for embolotherapy, while 3 other patients had smaller PAVMs. All clinical data are shown in Table 1. Calculations of the
Discussion
Unexplained dyspnea, cyanosis, or reduced physical capacity may be the only clues to identify individuals with PAVM. Establishing the diagnosis of PAVM is important in both symptomatic and asymptomatic individuals because treatment will reduce the risk of paradoxical embolus and cerebral abscess, and improve the well-being of symptomatic individuals.11011 Treatment with embolotherapy is recommended if the diameter of the PAVM feeding vessel is > 3 mm.12 Various screening protocols from
Conclusion
Screening for PAVMs among HHT patients is highly recommended because a PAVM may cause potentially hazardous complications. A screening protocol should be able to safely identify all PAVMs, with the smallest possible number of patients proceeding to extensive investigations (PA in this study). We have evaluated different screening procedures and conclude that CE followed by measurement of Pao2 after breathing 100% oxygen fulfills these requirements. However, a high cut-off value (500 mm Hg) for
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Supported by the Danish Medical Association Research Foundation and Ingemann O’Buchs Foundation.